Assessment of objective visual function following idebenone administration in patients with leber hereditary optic neuropathy.

Autor: Masuda Y; Department of Ophthalmology, The Jikei University School of Medicine, 3-25-8 Nishi-Shinbashi Minato-ku, Tokyo, 105-8461, Japan. ymasuda@jikei.ac.jp., Ishikawa H; Department of Ophthalmology, Hyogo Medical University, Nishinomiya, Hyogo, Japan.; Department of Ophthalmology, Mirai Eye and Skin Clinic, Joto-ku, Osaka, Japan., Ishikawa H; Department of Ophthalmology, Kitasato University School of Medicine, Sagamihara, Kanagawa, Japan., Kezuka T; Department of Ophthalmology, Tokyo Medical University, Nishi‑Shinjuku, Shinjuku‑ku, Tokyo, Japan., Miyazaki A; Brain Science Institute, Tamagawa University, Tokyo, Japan., Matsumoto K; Brain Science Institute, Tamagawa University, Tokyo, Japan., Gomi F; Department of Ophthalmology, Hyogo Medical University, Nishinomiya, Hyogo, Japan., Mimura O; Department of Ophthalmology, Hyogo Medical University, Nishinomiya, Hyogo, Japan., Shikishima K; Department of Ophthalmology, The Jikei University School of Medicine, 3-25-8 Nishi-Shinbashi Minato-ku, Tokyo, 105-8461, Japan., Nakano T; Department of Ophthalmology, The Jikei University School of Medicine, 3-25-8 Nishi-Shinbashi Minato-ku, Tokyo, 105-8461, Japan., Terao M; Research Institute for Time Studies, Yamaguchi University, Yamaguchi, Japan.
Jazyk: angličtina
Zdroj: Japanese journal of ophthalmology [Jpn J Ophthalmol] 2024 Jul 05. Date of Electronic Publication: 2024 Jul 05.
DOI: 10.1007/s10384-024-01077-z
Abstrakt: Purpose: To objectively assess visual function in Leber's Hereditary Optic Neuropathy (LHON) patients; this study evaluated pre- and post-idebenone treatment changes in primary visual cortical (V1) responses using functional magnetic resonance imaging (fMRI), given the challenges in subjective testing due to central retinal ganglion cell damage.
Study Design: A descriptive study involving four confirmed LHON patients.
Methods: Four patients received 900 mg/day of oral idebenone for 24 weeks. Baseline and post-treatment visual acuity, visual fields, and BOLD fMRI responses while passively viewed drifting contrast pattern visual stimuli were compared with self-reported symptoms.
Results: Post-idebenone, one patient showed positive trends across subjective tests, reported symptoms, and fMRI. Two patients had stable symptoms and fMRI responses; one improved on subjective tests, and another worsened slightly. Another patient improved in visual field tests despite worsening symptoms and fMRI trends.
Conclusion: fMRI may offer a valuable objective measure of visual functions in LHON and appears to be more relevant in assessing symptoms. Further research with more participants is needed to ascertain fMRI's role in developing objective visual assessments and treatment evaluation.
(© 2024. Japanese Ophthalmological Society.)
Databáze: MEDLINE
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